Paraneoplastic cerebellar degeneration: Auto-antibodies cause damage to Purkinje cells by changing the calcium homeostasis

Abstract

Paraneoplastic cerebellar degeneration (PCD) is a paraneoplastic neurological syndrome that is characterised by non-reversible damage of Purkinje cells. PCD usually affects women with gynaecological cancer and symptoms like diplopia, vertigo and ataxia develop within weeks to months. The patient-serum and cerebrospinal fluid contain immunoglobulin G anti-Yo-antibodies that react with cytoplasmic components of targeted Purkinje cells. Previously, we have shown that the anti-Yo-antibodies are directed against human cerebellar degeneration-related antigens (CDR2 and CDR2L) and cause cell death if they are taken up by viable Purkinje cells, and accumulate intracellularly.